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Mol. Cells 2010; 29(1): 63-69

Published online January 31, 2010

https://doi.org/10.1007/s10059-010-0017-z

© The Korean Society for Molecular and Cellular Biology

Downregulation of Wnt-Mediated ROS Generation Is Causally Implicated in Leprechaunism

Ji Won Park1,4, Hye Sun Kuehn1,4, So Youn Kim1, Kyung Min Chung1, Hyun Choi1, Mira Kim1, Jaesang Kim1, Soo Young Lee1, Duk Soo Bae2, Dong-Kyu Jin3, and Yun Soo Bae1,*

1Department of Life Sciences, Ewha Womans University, Seoul 120-750, Korea, 2Department of Obstetric and Gynecology, Samsung Hospital, Seoul 135-710, Korea, 3Department of Pediatrics, Samsung Hospital, Seoul 135-710, Korea, 4These authors contributed equally to this work.

Correspondence to : *Correspondence: baeys@ewha.ac.kr

Received: July 16, 2009; Revised: October 8, 2009; Accepted: October 14, 2009

Abstract

Although mutations in the insulin receptor have been causally implicated with leprechaunsim, the full pathophysiology of the syndrome cannot be accounted for by malfunction of this gene alone. We sought to characterize a connection between Wnt-mediated cell signaling and the production of reactive oxygen species (ROS) which revealed a novel mechanistic basis for understanding the pathogenesis of leprechaunism. To identify candidate genes involved in this process, a PCR-based subtractive hybridization was performed. Candidate genes were examined for interaction with the Wnt signaling pathway and ROS generation. We found that Dickkopf 1 (Dkk1), a Wnt inhibitor, is overexpressed in skin fibroblast cells derived from three leprechaunism patients and that the cells showed an impaired response to Wnt2 in terms of

Keywords dickkopf, leprechaunism, NADPH oxidase, NF-AT, Wnt

Article

Research Article

Mol. Cells 2010; 29(1): 63-69

Published online January 31, 2010 https://doi.org/10.1007/s10059-010-0017-z

Copyright © The Korean Society for Molecular and Cellular Biology.

Downregulation of Wnt-Mediated ROS Generation Is Causally Implicated in Leprechaunism

Ji Won Park1,4, Hye Sun Kuehn1,4, So Youn Kim1, Kyung Min Chung1, Hyun Choi1, Mira Kim1, Jaesang Kim1, Soo Young Lee1, Duk Soo Bae2, Dong-Kyu Jin3, and Yun Soo Bae1,*

1Department of Life Sciences, Ewha Womans University, Seoul 120-750, Korea, 2Department of Obstetric and Gynecology, Samsung Hospital, Seoul 135-710, Korea, 3Department of Pediatrics, Samsung Hospital, Seoul 135-710, Korea, 4These authors contributed equally to this work.

Correspondence to:*Correspondence: baeys@ewha.ac.kr

Received: July 16, 2009; Revised: October 8, 2009; Accepted: October 14, 2009

Abstract

Although mutations in the insulin receptor have been causally implicated with leprechaunsim, the full pathophysiology of the syndrome cannot be accounted for by malfunction of this gene alone. We sought to characterize a connection between Wnt-mediated cell signaling and the production of reactive oxygen species (ROS) which revealed a novel mechanistic basis for understanding the pathogenesis of leprechaunism. To identify candidate genes involved in this process, a PCR-based subtractive hybridization was performed. Candidate genes were examined for interaction with the Wnt signaling pathway and ROS generation. We found that Dickkopf 1 (Dkk1), a Wnt inhibitor, is overexpressed in skin fibroblast cells derived from three leprechaunism patients and that the cells showed an impaired response to Wnt2 in terms of

Keywords: dickkopf, leprechaunism, NADPH oxidase, NF-AT, Wnt

Mol. Cells
Aug 31, 2022 Vol.45 No.8, pp. 513~602
COVER PICTURE
Cryo-EM structure of human porphyrin transporter ABCB6 (main figure) shows that binding of hemin (inset, magenta) in concert with two glutathione molecules (cyan) primes ABCB6 for high ATP turnover (Kim et al., pp. 575-587).

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